The aim of this review is to demonstrate the spectrum of conditions encompassed by the term 'chronic progressive cicatrising conjunctivitis', to discuss mechanisms of conjunctival scar tissue formation and to describe the sequelae and therapeutic options in this potentially blinding condition. Chronic progressive cicatrising conjunctivitis is found in association with some mucocutaneous disorders (cicatricial pemphigoid, linear IgA disease), as part of paraneoplastic syndromes and after long-term treatment with certain systemic and topical medications (pseudo-pemphigoid). Recent studies on the conjunctiva of pemphigoid patients indicate that macrophages may play a pivotal role in chronic progressive conjunctival cicatrisation. They mediate the transition from inflammation to scar tissue by secretion of fibrogenic cytokines. There is evidence that similar mechanisms are involved in the other fibrosing conjunctival disorders. Sequelae of chronic conjunctival cicatrisation include the obstruction of lacrimal and meibomian glands, tear film alterations, trichiasis, keratopathy and blindness. Present possibilities and future options for the treatment of this condition are discussed.